Report the unusual MRI brain findings and outcome after treatment with maintenance dose of intravenous immunoglobulin (IVIG) in MFS with a delayed bilateral facial nerve palsy.

MFS is defined by the clinical triad of ataxia, ophthalmoplegia, and areflexia. There is limited data on the efficacy of repeated doses of IVIG and imaging characteristics in MFS with a delayed bilateral facial nerve palsies.

20 year-old African American female presented with a four day history of left eyelid heaviness, horizontal diplopia, and gait instability. Examination was significant for dysarthria, near complete ophthalmoplegia, upper and lower extremity hyperesthesia, intraoral hyperesthesia, arreflexia, and ataxia. Cerebrospinal fluid studies demonstrated elevated protein (60 mg/dL), no nucleated cells, and glucose of 61 mg/dL. She was diagnosed with MFS and underwent treatment with IVIG 2g/kg. On day ten, she developed right sided facial droop, which quickly progressed to near total facial paralysis bilaterally, despite her presenting symptoms improving. MRI brain revealed enhancement of cranial nerves III and VII bilaterally. The patient was subsequently treated with maintenance IVIG 1g/kg every four weeks after the initial loading dose. GQ1B antibody titers on days three and ninety-one demonstrated titers of 1:12800 and 1:300, respectively. Serial facial nerve conduction studies on days one, fourteen, and seventy-five revealed normal, absent, and near normal responses, respectively. Clinically, the patient had near complete recovery in eighty-five days of her initial symptoms.

The patient demonstrated delayed onset of bilateral facial nerve palsies with corresponding MRI findings of enhancement of multiple cranial nerves.